Neurofibromatosis type 1 (NF-1) is a rare genetic disorder that affects 1 out of every 3000 live births.[1] NF-1 is an autosomal-dominant neurocutaneous disorder that affects the skin, bones, and nervous system.[1,2] Although kidney involvement is rare, it most commonly presents as renal artery stenosis,[3,4] which is reported in less than 1% of cases.[3] Glomerulonephritis is rare in NF-1 patients; only a few cases of membranous nephropathy[5–7] and focal segmental glomerulosclerosis,[8–10] and 3 of immunoglobulin A nephropathy (IgAN),[11,12] have been reported in NF-1 patients. The gene discussed is NF1; the disease is Renal artery stenosis.